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XPA deficiency affects the ubiquitin-proteasome system function.

Angélica Maria de Sousa Leal1, Lázaro Batista de Azevedo Medeiros1, Cesar Orlando Muñoz-Cadavid1

  • 1Departamento de Biologia Celular e Genética, Universidade Federal do Rio Grande do Norte, CEP 59072-970, Natal, RN, Brazil.

DNA Repair
|July 22, 2020
PubMed
Summary

Xeroderma pigmentosum complementation group A (XPA) deficiency impairs the ubiquitin-proteasome system

Keywords:
APE1NFE2L2Oxidative stressProteasomeProteolysisProteostasisTranscriptional regulationXPAXeroderma pigmentosum

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Area of Science:

  • Molecular Biology
  • Cell Biology
  • Genetics

Background:

  • Xeroderma pigmentosum complementation group A (XPA) is crucial for DNA repair, and its deficiency leads to cancer predisposition.
  • The precise mechanisms underlying XPA's role in cellular stress response and disease pathogenesis remain incompletely understood.
  • Oxidative stress significantly impacts cellular function and DNA integrity, exacerbating conditions linked to DNA repair deficiencies.

Purpose of the Study:

  • To investigate the transcriptional profile of XPA-deficient cells under oxidative stress.
  • To elucidate the role of the ubiquitin-proteasome system (UPS) and key regulatory factors in XPA-deficient cells.
  • To explore potential interactions between XPA, NFE2L2, and other relevant proteins in cellular stress response.

Main Methods:

  • Transcriptional profiling of XPA-deficient cells exposed to oxidative stress.
  • Co-immunoprecipitation assays to identify protein-protein interactions.
  • Analysis of protein expression, proteasome activity, and gene expression in XPA-deficient cells and C. elegans models.

Main Results:

  • XPA-deficient cells exhibit an imbalance in ubiquitin-proteasome system (UPS) gene expression under oxidative stress.
  • Nuclear factor erythroid 2-related factor-2 (NFE2L2) protein expression and proteasome activity are decreased in XPA-deficient cells.
  • Interactions between XPA, apurinic-apyrimidinic endonuclease 1 (APE1), and NFE2L2 were identified, with potential involvement of GADD45β.
  • Conservation of XPA and NFE2L2 interactions was observed in an RNAi-based C. elegans model.

Conclusions:

  • XPA-deficient cells activate stress responses but fail to properly engage the UPS cytoprotective mechanisms under oxidative stress.
  • Impaired UPS function in XPA deficiency may contribute to the characteristic phenotypes observed in xeroderma pigmentosum patients.
  • The findings highlight a critical role for XPA in maintaining UPS integrity and cellular protection against oxidative damage.